In the latest Case Record of the Massachusetts General Hospital, a 51-year-old woman presented with a history of recurrent epistaxis and oral ulcers that were transiently responsive to glucocorticoids. Examination revealed erosions of the mucous membranes of the tongue, soft palate, and buccal mucosa. A diagnostic procedure was performed.
Chronic, persistent multifocal ulcers in the oral cavity are seen in the following three broad categories of conditions: infections, immune-mediated disorders, and autoimmune blistering disorders. Some important differentiating features include the size and appearance of lesions, involvement of keratinized versus nonkeratinized sites (especially the gingiva), complete healing of lesions between outbreaks, and involvement of skin.
Many viral infections (e.g., hand, foot, and mouth disease and herpangina, caused by coxsackieviruses or enteroviruses) give rise to acute multifocal ulcerations in the oral cavity that are self-limiting; the persistent nature of this patient’s ulcers eliminates most of these. Viruses of the herpesvirus family establish latency and may cause recurrent oral or oropharyngeal ulcers. The most common culprit is herpes simplex virus (HSV). Recrudescent HSV infections frequently present as herpes labialis and, in immunocompetent patients, as ulcers on the keratinized mucosa of the gingiva, hard palate, and dorsum of the tongue.
Granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) is a necrotizing and granulomatous vasculitic disorder with involvement of the upper airways, lungs, and kidneys; patients with this and other ANCA-associated vasculitides may present with oral ulcers. Patients with a variant of granulomatosis with polyangiitis may present with limited involvement of the upper airways and oral cavity. However, the oral ulcers tend to be large and necrotic, and the gingiva typically shows hyperplasia with petechial hemorrhages (“strawberry gingivitis”). ANCAs with specificity for serine proteinase 3 (more common) or myeloperoxidase (less common) are almost always positive.
Morning Report Questions
Q: What is mucous-membrane pemphigoid?
A: Mucous-membrane pemphigoid is a subepithelial blistering disorder. Patients typically present with bright red, painful denudation on the gingiva (desquamative gingivitis) or with ulcers on the palate. Bleeding on tooth brushing is common. Collapsed bullae and erosions may also occur in other mucosal sites, although they are almost always accompanied by involvement of the gingiva or palate or both. Scarring is common in ocular pemphigoid but not in oral pemphigoid, and erosive lesions of the larynx, pharynx, and nasal mucosa occur in approximately 35% of cases. Erosive lesions may lead to scarring, stenosis, synechiae, obstruction, and dyspnea.
Q: What is oral pemphigus vulgaris?
A: Pemphigus vulgaris is an intraepithelial blistering disorder. Lesions begin in the oral cavity in up to 60% of patients, with skin lesions developing months or years later. The soft palate and the mucosa of the hard palate are commonly involved because of trauma from swallowing. Gingival manifestations are similar to those of mucous-membrane pemphigoid. Laryngeal or pharyngeal involvement is present in up to 80% of patients, nasal crusting and erosions are found in up to 60%, and the ears are involved in 20%.